Highly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells

Friedreich ataxia is an inherited neurodegenerative disease that leads to progressive disability. There is currently no effective treatment and patients die prematurely. The underlying genetic defect leads to reduced expression of the mitochondrial protein frataxin. Frataxin insufficiency causes mit...

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Publicado en:Neurobiol Dis
Autores principales: Rufini, Alessandra, Cavallo, Francesca, Condò, Ivano, Fortuni, Silvia, De Martino, Gabriella, Incani, Ottaviano, Di Venere, Almerinda, Benini, Monica, Massaro, Damiano Sergio, Arcuri, Gaetano, Serio, Dario, Malisan, Florence, Testi, Roberto
Formato: Artigo
Lenguaje:Inglês
Publicado: Academic Press 2015
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC4358773/
https://ncbi.nlm.nih.gov/pubmed/25549872
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2014.12.011
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